History of dislike for sweet food items is typically present in:
Correct Answer: Hereditary fructose intolerance
Description: Ans. is 'c' i.e. Hereditary fructose intoleranceRef.: Harrison 17th/e p 2458 & 16th/e p 2321 (15th/e p2289) In hereditary fructose intolerance patients are healthy and asymptomatic until fructose or sucrose (table sugar) is ingested. With the ingestion of sugar severe clinical symptoms appear. Which are -JaundiceHepatomegalyVomitingLethargyIrritabilityConvulsionsProteinuriaHereditary fructose intolerance is caused by the deficiency of Aldolase B.Aldolase B catalyzes the following reactionFructose - 1 - phosphate Aldolase B Glyceraldehyde* Dihydroxyacetone phosphate Deficiency of this enzyme causes rapid accumulation of fructose -1- phosphate and initiates severe toxic symptoms when exposed to fructose.Diagnosis -It is diagnosed by the presence of reducing substance (fructose) in the urine.The appearance of hypoglycemia and hypophosphatemia after fructose (200 mg/kg) loading is diagnosticTreatment:Treatment consists of eliminating fructose from the dietAs affected individuals grow up they may recognize the association of nausea and vomiting with fructose containing foods and selectively avoids them
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